What Is Ewing Sarcoma?

Ewing sarcoma is a cancerous tumor that can happen in any bone in the body, but most often happens in bones of the arms, legs, rib, spine and pelvis. Occasionally, a tumor can develop outside of a bone in the soft tissue around it.

Who Gets Ewing Sarcoma?

Most cases happen in teens and young adults (10 to 20 years old) and Ewing sarcoma affects more males than females.

What Are the Signs & Symptoms of Ewing Sarcoma?

Someone with Ewing sarcoma might have:

• a lump in the bone of the arm or leg
• pain in a bone
• swelling and warmth near a bone
• bone pain that does not get better over time or lasts longer than expected for a minor injury
• limping

If the cancer spreads, or metastasizes, it usually goes to the lungs, other bones, or to the bone marrow (the spongy material inside the bone).

How Is Ewing Sarcoma Diagnosed?

To diagnose Ewing sarcoma, a doctor will do an exam, take a detailed , and order some tests.

Tests can include:

• X-rays
• MRI (magnetic resonance imaging)
• a bone scan
• a biopsy (getting a sample of the tumor)

If the doctor diagnoses Ewing sarcoma, the child will have other imaging tests to see if the cancer has spread to any other part of the body. Doctors will also order blood tests to make sure that organs like the heart, liver, and kidneys are working well enough to tolerate treatment.

How Is Ewing Sarcoma Treated?

How doctors treat Ewing sarcoma depends on: 

• the size of the tumor
• the location of the tumor
• whether the disease has spread to other parts of the body

The types of treatment used include some or all of these:

Chemotherapy:Chemotherapy often is given first to shrink the tumor, improve pain, and stop the spread of cancer. Doctors also may use chemotherapy after radiation therapy or surgery to prevent the cancer cells from coming back.

Chemotherapy usually lasts 6 months to a year. During treatment, the child will usually stay in the hospital for a 3–6 days every 2 to 3 weeks. Some kids may need to return to the hospital between treatments if they have side effects, like fever or infection, or if they need a blood transfusion.

After chemotherapy, the doctor's goal is to continue to kill tumor cells around the tumor. This can be done with:

Radiation therapy: High-energy X-rays are directed at the tumor to kill cancer cells. Doctors may use radiation to shrink the tumor before surgery. They may also use it instead of surgery if the tumor's location makes surgery difficult and might lead to long-term problems.

Surgery: The surgeon removes the tumor by cutting it out and trying to remove all of the tissue around the tumor that contains cancer cells. The goal is to have "negative margins" (this means cancer cells are not seen under the microscope in the tissue from the area outside the tumor).

Doctors sometimes do reconstructive surgery to rebuild the area where the tumor and other tissue was. They can do this with:

• a live tissue graft (from the patient's own body)
• donor tissue (usually from a bone bank)
• artificial tissue (made from metal or other synthetic material)

Sometimes, to make sure they remove all the cancer, doctors might need to amputate the affected limb (surgically removing all or part of it along with the tumor). A prosthesis (artificial arm or leg) can replace what was removed. In some cases, amputation offers the best chance for cure.

What Are the Side Effects of Treatment?

Complications after surgery can include:

• infection
• slow healing of the wound

Chemotherapy-related side effects can include:

• short-term problems: anemia (low red blood count); bleeding easily; risk of infection; risk of kidney, liver, or heart damage
• long-term problems: possible increased risk of developing other cancers; fertility problems

Amputation-related problems can include:

• an adjustment period while the child learns to learn to use a prosthetic
• long-term psychological and social issues related to the prosthesis
• fracture or poor healing of the bank bone

Looking Ahead

Ewing sarcoma has the best chance of being cured when it's treated by experts in pediatric cancer treatment. Young adults may benefit from treatment at a children's hospital instead of an adult hospital because of the care team's expertise.

Successful treatment is harder if the disease spreads to other parts of the body. But with the combination of treatments available — and powerful new therapies on the horizon — the outlook for kids with advanced disease is improving.

Having a child being treated for cancer can feel overwhelming for any family. But you're not alone. To find support, talk to anyone on the care team or a hospital social worker. Many resources are available to help you and your child. You also can find information and support online at:

• National Cancer Institute
• American Childhood Cancer Organization
• OncoLink